Hein J
Z Erkr Atmungsorgane. 1985;164(1):19-24.
In 1967 Spock et al. reported on the serum of cystic fibrosis (CF) homozygotes containing a factor altering the coordination of ciliary motion in rabbit tracheal explants. Just in 1967 Mangos et al. found sweat and saliva from CF homozygotes having an inhibitory effect on sodium reabsorption in the rat parotid gland. Since that time the existence of CF specific humoral factors was supposed. Hitherto mainly biological tests (especially tests of ciliary dyskinesia) were used to prove these factors. These tests caused different results which even were doubtful with regard to the existence of CF specific proteins. Recently it is possible to differentiate between proteins with effects of ciliary dyskinesia and a CF specific protein by means of high sensitive biochemical and immunological methods of protein distinction. In future one can expect elucidation of question related to the importance of CF protein in pathogenesis and diagnosis of cystic fibrosis.
1967年,斯波克等人报告称,囊性纤维化(CF)纯合子的血清中含有一种因子,可改变兔气管外植体中纤毛运动的协调性。同年,曼戈斯等人发现,CF纯合子的汗液和唾液对大鼠腮腺中的钠重吸收有抑制作用。从那时起,人们就推测存在CF特异性体液因子。迄今为止,主要通过生物学测试(尤其是纤毛运动障碍测试)来证明这些因子。这些测试产生了不同的结果,甚至关于CF特异性蛋白质的存在也存在疑问。最近,通过高灵敏度的蛋白质区分生化和免疫方法,可以区分具有纤毛运动障碍作用的蛋白质和CF特异性蛋白质。未来,人们有望阐明与CF蛋白在囊性纤维化发病机制和诊断中的重要性相关的问题。